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[Adult Still's disease].
Nihon Rinsho. Japanese Journal of Clinical Medicine
|March 17, 1999
Summary
Adult onset Still's disease is a systemic inflammatory condition. Severe cases may involve complications like disseminated intravascular coagulation, requiring treatments beyond NSAIDs.
Area of Science:
- Rheumatology
- Internal Medicine
- Immunology
Context:
- Adult onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder.
- Characterized by fever, rash, and joint pain, its etiology remains unknown.
- While often benign, severe manifestations can occur, impacting multiple organ systems.
Purpose:
- To review the clinical presentation, diagnosis, and management of Adult onset Still's disease.
- To highlight severe complications and treatment strategies for refractory cases.
Summary:
- AOSD presents with characteristic fever, rash, and arthritis.
- Severe cases may include disseminated intravascular coagulation (DIC), hemophagocytosis, amyloidosis, and respiratory failure.
- Treatment involves NSAIDs, with corticosteroids (prednisolone) and DMARDs/immunosuppressants for severe or refractory disease.
Impact:
- Provides an overview of AOSD for clinicians.
- Emphasizes the importance of recognizing and managing severe complications.
- Informs treatment decisions for challenging AOSD cases.